A very rare genetic condition, progeria is characterised by accelerated ageing in children who typically die of heart disease caused by complications at an average age of 14 years.
In a study published in the journal Nature Communications, a team of Swedish and Italian scientists revealed a new possible treatment option for fighting progeria.
Researchers from Sweden’s Karolinska Institute and the FIRC Institute of Molecular Oncology in Italy specifically focused on how antisense oligonucleotide therapies could be used in fighting the very rare genetic fatal disease which affects about one in 18 million children, leading to premature aging and death in adolescence from complications of cardiovascular disease.
Hutchinson-Gilford Progeria Syndrome
It was Eriksson who identified in 2003 the mutation which inhibits cell division related to progeria, also known as Hutchinson-Gilford progeria syndrome, which is linked to progerin, a defect form of the lamin A protein found in the cell nucleus.
She was echoed by Agustin Sola-Carvajal, former postdoc in Eriksson’s research group and co-author of the study, who explained that “in a gene altered mouse model of progeria treated in the same way, we saw a significant increase in both the maximum life expectancy, up 44 percent, and the average life expectancy, up 24 percent”.
Man with Progeria Syndrome
He touted the results as “very promising”, adding that more research is needed to assess “how the relatively low levels of progerin seen in healthy individuals contribute to ageing and age-related disease”.
The study comes after more than a dozen treatments of progeria have been tested over the past few years, with no tangible results related to clinical trials conducted in patients suffering from the disease.
The new treatment pertains to researchers managing to reduce the level of telomeric non-coding ribonucleic acid (RNA) by adding antisense oligonucleotides to inactivate harmful genes.